Autoimmune bullous dermatoses.
J Dtsch Dermatol Ges
; 21(4): 405-412, 2023 04.
Article
en En
| MEDLINE
| ID: mdl-37070500
ABSTRACT
Autoimmune bullous dermatoses (AIBD) are a heterogeneous group of about a dozen diseases characterized clinically by erosions and blisters and immunopathologically by autoantibodies against structural proteins of the skin or transglutaminase 2/3. The diagnosis of AIBD has made tremendous progress in the last decade due to the availability of standardized serological assays that, knowing the clinical picture, allow the diagnosis in the vast majority of patients. The development of various in vitro and in vivo models of the most common AIBD, namely, bullous pemphigoid, pemphigus vulgaris, mucous membrane pemphigoid, and the rare epidermolysis bullosa acquisita, allows identification of key molecules and inflammatory pathways as well as preclinical evaluation of the effect of new anti-inflammatory agents. The approval of rituximab for moderate and severe pemphigus vulgaris and the development of national and international guidelines for the most common AIBD have considerably advanced the care of these patients. Nevertheless, the limited therapeutic armamentarium is the main challenge for the management of AIBD. Several phase II and III randomized controlled clinical trials provide hope for new, effective, and safe therapeutic options in the coming years. This review summarizes the epidemiology, clinic, diagnosis, pathophysiology, and therapy of AIBD and gives an outlook on both current diagnostic and therapeutic needs as well as future developments.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Enfermedades Autoinmunes
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Enfermedades Cutáneas Vesiculoampollosas
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Penfigoide Ampolloso
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Pénfigo
Tipo de estudio:
Clinical_trials
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Diagnostic_studies
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Guideline
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Prognostic_studies
Límite:
Humans
Idioma:
En
Año:
2023
Tipo del documento:
Article